Perioperative Nutritional Status and Organ Dysfunction Following Surgery for Congenital Heart Disease.

Children with congenital heart disease (CHD) are at risk of malnutrition; however, there is limited information regarding the impact of nutritional status on organ dysfunction and outcomes after surgery for CHD. The study aim was to assess the association between malnutrition, organ dysfunction, and outcomes after surgery for CHD. Retrospective cohort study of patients aged 30 days to 18 years admitted to the cardiac intensive care unit (CICU) following cardiac surgery. Nutritional status (malnutrition defined as weight for age z-score < - 2) and validated organ dysfunction scores (pSOFA and PELOD-2) on CICU days 1 and 3 were collected. The cohort included 967 patients with a median age of 2.8 years (IQR 0.46, 7.12) and hospital survival of 98.86%. The prevalence of malnutrition was 18.5% (n = 179). By multivariable logistic regression analysis including age, malnutrition, cardiopulmonary bypass time, and duration of mechanical ventilation; High STAT category (OR 7.51 [1.03-54], p = 0.0462) and PSOFA score > 5 day 1 (OR 1.84 [1.25-2.72], p = 0.0021) were associated with mortality; in a similar model including the same variables; High STAT category (OR 9.12 [1.33-62], p = 0.0243) and PELOD-2 score > 5 day 1 (OR 1.75 [1.10-2.77], p = 0.0175) were associated with mortality. Malnutrition was associated with persistent or worsening organ dysfunction by pSOFA (p < 0.05) and PELOD-2 (p < 0.01) on day 3. Malnutrition was present in infants and children undergoing surgery for congenital heart disease. Organ dysfunction and high surgical risk were associated with mortality. Malnutrition was not associated with mortality but was associated with postoperative organ dysfunction.

SNARE-ing the Reason for Post-Cardiac Surgery Critical Illness-Related Corticosteroid Insufficiency.

Critical illness-related corticosteroid insufficiency (CIRCI) can cause hemodynamic instability in neonates after congenital heart surgery with manifestations that increase morbidity and potential mortality. We retrospectively reviewed neonates who underwent cardiac surgery between August 2018 and July 2020 at a freestanding children's hospital, had next-generation sequencing performed, and had their cortisol levels drawn as standard clinical care after cardiac surgery. The groups were defined as CIRCI (with a cortisol level ≤ 4.5 mcg/dL) and non-CIRCI (level > 4.5 mcg/dL). The CIRCI group (n = 8) had a 100% incidence of heterozygous gene mutation on STX1A with splicing or loss of function, and this mutation was not found in the non-CIRCI group (n = 8). Additional gene mutations were found in the CIRCI group on RAB6A, ABCA3, SIDT2, and LILRB3, with no incidence in the non-CIRCI group. Three additional mutations were found across the CIRCI group in INPPL1 and FAM189A2 (both splicing and missense), with 12-25% of patients in the non-CIRCI group also displaying these mutations. Novel genetic abnormalities were seen in neonates with symptoms of CIRCI with potential cardiac implications from a gene mutation for STX1A. Compounding effects of additional gene mutations need to be confirmed and explored for potential predisposition to hemodynamic instability during times of stress.

A methodological and clinical approach to measured energy expenditure in the critically ill pediatric patient.

The metabolic response to injury and stress is characterized initially by a decreased energy expenditure (Ebb phase) followed by an increased metabolic expenditure (Flow phase). Indirect calorimetry is a methodology utilized to measure energy expenditure and substrate utilization by measuring gas exchange in exhaled air and urinary nitrogen. The use of indirect calorimetry in critically ill patients requires precise equipment to obtain accurate measurements. The most recent guidelines suggested that measured energy expenditure by indirect calorimetry be used to determine energy requirements. This article reviews the methodological and clinical use of indirect calorimetry in critically ill pediatric patients.

Ventricular Tachycardia in Fabry Disease Detected in a 50-Year-Old Woman during 14-Day Continuous Cardiac Monitoring.

Fabry disease is an X-linked lysosomal storage disorder. Female carriers were long thought to be asymptomatic; however, research has revealed the opposite. Cardiac conditions are the chief causes of death in women with Fabry disease. Although ventricular tachycardia has been reported in male patients with Fabry disease, it is not thought to be a frequent finding in females. We describe the case of a 50-year-old woman in whom we used 14-day continuous electrocardiographic monitoring to identify nonsustained ventricular tachycardia, after electrocardiograms and 24-hour Holter monitoring failed to detect the arrhythmia. A permanent implantable cardioverter-defibrillator relieved the patient's symptoms. We discuss why this case supports the need for more extensive electrophysiologic evaluation in women who have Fabry disease.